scholarly journals Characterization of A Rare Case of Vulvar Epithelioid Sarcoma with local recurrence and metastases

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S81-S81
Author(s):  
E Shkolnik ◽  
D Cai

Abstract Introduction/Objective Epithelioid sarcoma (ES) is a rare, malignant mesenchymal neoplasm that has a known tendency for local recurrence, regional lymph node involvement, and distant metastases. Two histologic variants have been recognized: classic ES also known as the distal type, and proximal-type ES (PES). The classic ES is common in young adults. It occurs more frequently in the distal upper extremities followed by the distal lower limbs and has a male prevalence of 2:1. Conversely, PES commonly involves deep tissues in the pelvic region, including the genital area. It tends to occur in older patients and follows a more aggressive clinical course. In the female genital tract, PES occurs most frequently in the vulva. The incidence of primary sarcoma of the vulva accounts for 1.5-5% of all malignant tumors, making PES a very rare incidence. Methods/Case Report Here we report a 60-year-old female diagnosed with vulvar epithelioid sarcoma treated with a right radical vulvectomy and bilateral inguinal lymph node dissection in 2008. In 2017, further surgery and adjuvant radiation were given for local recurrence. In 2020, the patient developed left hip pain and was found to have an expansile lytic lesion in the left proximal femur. Extensive resection was performed. Grossly the vulvar lesion was nodular with diffuse hemorrhage, degeneration, and necrosis. Microscopically, the tumor cells had large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Histologically, the morphology of the tumor cells are similar for the primary vulvar specimen and the bone metastases. Immunohistochemically, the tumor cells are positive for vimentin, GATA, FLI-1, SMA, SMHC, partially positive for CAM5.2, AE1/AE3, CD31, and CD163. Immunohistochemistry was negative for CDX2, CD56, S-100, TTF-1, CK5/6, CK20, P40, mammoglobin, MOC31, ER, CK7, CK903, HMB45, PAX8. A Ki-67 proliferative index was around 30-40%. NGS molecular testing detected a SMARCB1 mutation with loss of exons 1-3 and exons 7-9 supporting the diagnosis of epithelioid sarcoma. Results (if a Case Study enter NA) NA Conclusion In summary, we report a case of PES of the vulva in a 60 year old female. Grossly, the lesion was nodular with histology showing large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It showed loss of INI1/SMARCB1 nuclear expression. The patient is receiving further adjuvant treatment and shows no new metastases.

2020 ◽  
Vol 28 (6) ◽  
pp. 691-695 ◽  
Author(s):  
Irene Y. Chen ◽  
Bushra G. Fazili ◽  
Xiaoyan Liao

Glomus tumor is a rare mesenchymal neoplasm originating from the modified smooth muscle cells of the glomus body. Primary colonic glomus tumor is extremely rare with only 5 cases published in the English literature. In this article, we report the sixth case of primary colonic glomus tumor in a 50-year-old female with no significant past medical history who presented with routine screening colonoscopy. The entire colon was endoscopically unremarkable except an incidental 6-mm sessile polyp located in the descending colon. Biopsy showed a densely cellular neoplasm composed of small, bland, slightly spindled to predominantly epithelioid cells with clear to eosinophilic cytoplasm arranged in nests and sheets. The tumor cells were interspersed with slit-like thin-walled vessels and scattered short nerve bundles. Immunohistochemically, the tumor cells were positive for smooth muscle actin, h-caldesmon, and CD34 (focal), but completely negative for HMB45, S100, EMA, desmin, DOG-1, and CD117. The histologic features and immunohistochemical profile supported a diagnosis of primary colonic glomus tumor. The patient was asymptomatic and disease free after the procedure.


2019 ◽  
pp. 43-51
Author(s):  
Anthony Kodzo-Grey Venyo

Less than 50 cases of Primary Leiomyosarcomas of the Penis (PLOP) have been reported despite this PLOPs are the second most common sarcomas of the penis. The usual site for the development of PLOP is the shaft or base of the penis but the malignancy can affect any site of the penis. PLOPs are likely to be superficially located above the tunica albuginea in comparison with deep seated PLOPs. Superficial PLOPs may be asymptomatic or they may present as lumps, ulcers or nodules on the penis. Deep PLOPs may manifest with dysuria and difficulty with micturition when they compress the urethra. Diagnosis of PLOP is based upon the pathology examination features of specimens of the penile lesion that show interlacing fascicles of spindled-cells that have abundant eosinophilic cytoplasm, focal juxta-nuclear vacuoles and blunt ended nuclei and moderate to severe nuclear atypia, rarely atypical multi-nucleated giant cells and focal necrosis tend to be seen. There may be evidence of many mitoses. Immunohistochemistry studies of PLOP tend to show tumor cells that exhibit positive staining for desmin, muscle specific-actin, and alpha smooth muscle actin. Most often superficial PLOPs tend to be treated by wide complete excision of the tumor with tumor free and clear surgical resection margins. Deep seated PLOPs have also been treated by complete excision in various forms depending upon the site and size of the tumor and some of these have included Wide excision, partial penectomy and total penectomy alone and sometimes with adjuvant chemotherapy/radiotherapy. Some cases of deep PLOPs tend to be associated with the development of recurrences and metastases as well as death of the patient and hence deep PLOPs are aggressive tumors. Some people are of the opinion that chemotherapy and radiotherapy are not effective for the adjuvant treatment of PLOP. For this reason it would appear that there is need for the development of new chemotherapy medicaments that would effectively destroy PLOP tumor cells. There is need to undertake a global multi-center treatment trial of immunotherapy, chemotherapy and radiotherapy in order to streamline the treatment of PLOPs especially deep PLOPs.


2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Jing Yang ◽  
Lei Dong ◽  
Hong Du ◽  
Xiu-bo Li ◽  
Yan-xiao Liang ◽  
...  

Abstract Background Translocation-associated renal cell carcinoma involving ALK (ALK-tRCC) is a rare subtype of adult renal cell carcinoma (RCC) reported in recent years. It was recognized as a group of emerging /provisional RCC in the latest World Health Organization’s classification (2016). Case presentation A new Chinese case of ALK-tRCC was reported. The patient was a 58-year-old man with a tumor in kidney. The tumor was composed of sheets of large cells with abundant eosinophilic cytoplasm and indistinct cell borders but conspicuous intracytoplasmic vacuoles. The nuclei were enlarged with a nucleolar of grade 4. Immunohistochemically, tumor cells were diffusely positive for PAX8, keratin (AE1/AE3), epithelial membrane antigen (EMA) and CK7. Fluorescent in situ hybridization (FISH) showed a rearrangement of ALK in tumor cells. Conclusion ALK-tRCC is a rare subtype of adult RCC. Its diagnosis is very difficult because the histological spectrum is very wide. We suggested that RCCs should be screened for ALK expression by immunohistochemistry (IHC) for the patient might benefit from ALK inhibitors therapy.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Valeria Pereira Martinez ◽  
Marilin Nicholson ◽  
Trushar Patel

Epithelioid sarcoma (ES) is a rare malignant mesenchymal neoplasm that accounts for less than one percent of all soft-tissue sarcomas. Only two cases of ES involving the adrenal gland were found after a literature review. We report a case of an 82-year-old female initially presenting with right flank pain who was subsequently found to have an incidental left adrenal mass on CT imaging. After appropriate diagnostic workup, the patient underwent surgical resection. A diagnosis of ES was made from the histopathological analysis. The characteristic findings of ES are epithelioid cells with rhabdoid morphology and moderate eosinophilic cytoplasm. Immunohistochemical findings are significant for positive staining for epithelial markers, cytokeratins, vimentin, and CD34, and loss of INI-1 stain. Due to the aggressive nature and limited data of ESs, the standard treatment continues to remain wide surgical excision.


2008 ◽  
Vol 11 (2) ◽  
pp. 142-147 ◽  
Author(s):  
Jean Luo ◽  
Steven Melnick ◽  
Anthony Rossi ◽  
Redmond P. Burke ◽  
John D. Pfeifer ◽  
...  

A case of primary alveolar soft part sarcoma (ASPS) of the heart is reported in an 11-year-old female as 1 of 16 cases of ASPS presenting in the first 2 decades of life in our institutional 17-year review period. The classic alveolar or organoid pattern was inconspicuous as compared to a more diffuse or formless pattern consisting of a population of uniform round cells with abundant eosinophilic cytoplasm, but in addition there was a second, minor population of gigantiform tumor cells with a variety of unusual shapes. Scattered tumor cells contained dense eosinophilic condensations in the cytoplasm. Other unusual features for ASPS in our case included a lymphohistocytic reaction and zonal necrosis. Immunohistochemistry revealed nuclear reactivity for TFE3, and the ASPL-TFE3 fusion transcript was identified by reverse-transcriptase polymerase chain reaction. The only other examples of ASPS involving the heart were 3 cases in the literature of metastatic disease from tumors arising in the soft tissues. This initial case of primary cardiac ASPS joins the list of other types of sarcomas in children that have been reported as primary neoplasms of the heart.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S58-S58
Author(s):  
S Zaheer ◽  
K Xu ◽  
F Shakil

Abstract Introduction/Objective Epithelioid Sarcoma is a rare malignant soft tissue neoplasm that is well known for local recurrence, regional lymph node involvement, and metastasis. It accounts for less than one percent of all sarcomas. Two subtypes of epithelioid sarcoma are described: the conventional or classic subtype and the proximal subtype, with the classic type being more common than the proximal type. We present a case of proximal epithelioid sarcoma involving the inguinal region, in which the patient presented with the history of weight loss for one year and a slow- growing inguinal mass for the last few months. The biopsy was done in an outside hospital and showed carcinoma of unknown origin. Imaging also failed to reveal any clear source of this mass. Methods The mass was resected and sent to pathology, where H&E staining and immunohistochemistry was done for diagnosis. Results The H&E sections of the left inguinal mass showed proliferation of large epithelioid cells with a moderate amount of cytoplasm, vesicular nuclear chromatin and prominent nucleoli. Mitosis was also seen. Necrosis was not identified. Immunohistochemical staining was done and revealed that the tumor cells were immunoreactive for epithelial marker cytokeratin AE1/AE3 and showed loss of INI-1. Tumor cells were also negative for Myogenin, ERG, HMB-45, MART-1, Myo-D1, S100, and SMA. Considering the cytomorphology and immunophenotypic findings the diagnosis of epithelioid sarcoma, proximal type was made. Conclusion Epithelioid sarcoma is a malignant mesenchymal neoplasm with epithelioid cytomorphology and phenotype. Among the two types of epithelioid sarcoma, the proximal type is reported about 50% less than the classic type and is associated with aggressive behavior and worse prognosis. The classic cytomorphology and immunohistochemistry findings helped us in making the diagnosis in this case.


2011 ◽  
Vol 135 (2) ◽  
pp. 268-272 ◽  
Author(s):  
Jesse Hart ◽  
Srinivas Mandavilli

Abstract Epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy, most commonly arising in the deep soft tissues, but a variety of primary sites, including the adrenals, thyroid, skin, and bone, are encountered. On hematoxylin-eosin–stained sections, the pathologist encounters sheets of large, mildly to moderately pleomorphic epithelioid cells, with abundant eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Obvious vasoformative foci may not be present, creating confusion with metastatic carcinomas, malignant mesothelioma, melanoma, anaplastic lymphoma, epithelioid peripheral nerve sheath malignancies, and epithelioid sarcoma. Moreover, malignancies with apparent vascular differentiation must be distinguished from less aggressive vascular neoplasms, including epithelioid hemangioendothelioma. Given the range of clinical presentation, the diversity of primary sites, and the nonspecific initial histopathologic appearance, here we review the histologic findings and immunohistochemical profiles of epithelioid angiosarcoma and neoplasms in its differential diagnosis.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Shweta Agarwal ◽  

Papillary Thyroid Cancer (PTC) accounts for 80–90% of all thyroid malignancies [1]. The most common morphology is the classical papillary which has an indolent course. Aggressive variants exist, of which Tall Cell Variant (TCV) is the most common. TCV is defined as a PTC in which 30% or more of tumor cells are 2-3 times as long as they are wide [1,2]. The histology image (Figure 1A, magnification 4x) shows tumor cells arranged back to back with abundant eosinophilic cytoplasm, and typical nuclear features of PTC; namely overlapping enlarged nuclei, intranuclear grooves, and occasional pseudoinclusions. Figure 1B (magnification: 20x) shows a high power view of the same case where the tall cell features of individual tumor cells can be better appreciated (arrows). This important feature should be recognized and reported in pathology reports as TCVs exhibit worse clinical course with extensive lymph node metastasis, extra thyroidal extension and high rate of recurrence [1,2].


2002 ◽  
Vol 54 (6) ◽  
pp. 592-594 ◽  
Author(s):  
G.D. Cassali ◽  
F. Gärtner ◽  
F.C. Schmitt

This report describes the morphological and immunohistochemical findings of a case of mammary gland pleomorphic lobular carcinoma occurring in the canine species. Histologically, it was characterized by the presence of tumor cells loosely dispersed in the stroma or arranged in a linear pattern showing relatively abundant eosinophilic cytoplasm with an eccentric nuclei. These morphological features, characteristic of pleomorphic mammary gland lobular carcinoma in woman, were not previously described in dogs.


2018 ◽  
Vol 26 (7) ◽  
pp. 655-659 ◽  
Author(s):  
Masashi Miyaoka ◽  
Kazuhito Hatanaka ◽  
Masayuki Iwazaki ◽  
Naoya Nakamura

We report 2 cases of pulmonary adenocarcinoma mimicking desquamative interstitial pneumonia (DIP) with genetic analysis occurring in a 74-year-old woman and a 76-year-old woman. In both cases, the tumor was mainly composed of discohesive tumor cells, which filled and floated in the alveolar space in a DIP-like pattern. The tumor cells had abnormally large round to oval nuclei with fine chromatin and relatively abundant eosinophilic cytoplasm lacking pigmentation. Immunohistochemically, tumor cells in both cases were positive for CK7, TTF-1, napsin A, E-cadherin, β-catenin, and PD-L1 (one case had high expression and the other had low expression), and negative for CK5/6, CK20, p40, and ALK. However, the positive pattern of E-cadherin and β-catenin was incomplete on the circumference of the cell membrane in both cases and in one case, respectively. On genetic analysis, EGFR alteration (exon 21, L858R mutation) was observed in one case and ALK translocation was not observed in either. To the best of our knowledge, this is the first report of pulmonary adenocarcinoma mimicking DIP with genetic analysis.


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